Neurology

51. A 25-year-old woman is brought to the accident and emergency department after suffering a blackout. She was waiting for the bus when she felt nauseated, dizzy and sweaty. She lost consciousness for 1 minute. A friend says she was pale, had some jerking of her limbs for 10 s, but did not bite her tongue and there was no history of urinary incontinence. She recovered immediately with no confusion or disorientation. Examination is normal. ECG is normal. What further investigations are required to made a diagnosis? CT brain Doppler of carotids EEG MRI brain No investigations

Your answer

The most likely diagnosis is vasovagal syncope. It is usually preceded by symptoms of dizziness, feeling hot, nausea and darkening of vision. The syncope only lasts for a short period (up to a minute). Commonly there is some jerking of the limbs due to brain hypoperfusion. Tongue biting does not occur. Patients recover quickly after the syncope. Common predisposing factors are pain, prolonged standing, a hot environment and a heavy meal. No investigations are needed apart, perhaps, from an ECG in typical cases.

52. A 69-year-old man with recently diagnosed coeliac disease presents with paraesthesias in the feet and gait disturbance. On lower limb examination he has increased tone and weakness predominantly affecting the flexor muscle groups. Knee jerks are brisk and ankle jerks are absent. Both plantar responses are absent. He has loss of vibration sense to the costal margins and impaired jointposition sense at the toes, although pinprick and temperature sensations are normal. He has a macrocytic anaemia and his vitamin B12 level is confirmed to be very low. What imaging abnormalities are likely to be seen on spinal MRI? Diffuse patchy white-matter lesions on T2-weighted imaging Evidence of microhaemorrhages in the pyramidal tracts

Evidence of microhaemorrhages in the posterior columns Increased T1-weighted signal in the pyramidal tracts Increased T2-weighted signal in the posterior columns Your answer He has subacute combined degeneration of the cord due to vitamin B12 (cobalamin) deficiency. This causes the classic combination of a slowly evolving spastic paraparesis (long tract signs), peripheral neuropathy (absent ankle jerks) and a posterior column pattern of sensory loss (vibration and joint-position sense). The MRI typically shows increased signal on T2-weighted imaging, predominantly in the dorsal columns. This probably represents the known pathological changes of cord oedema and demyelination in the posterior and lateral cord. (These MRI features are, however, non-specific and can be seen in other conditions, eg multiple sclerosis.) Axonal loss and cord atrophy may develop in long-standing cases. However, the changes are partly or fully reversible in most cases with vitamin B12 replacement therapy.

53. A 34-year-old woman attends your clinic and gives a description of recurrent early morning attacks of severe throbbing unilateral orbital pain over the last 2 months. Each attack lasts 6090 minutes, but leaves a dull ache around the eye lasting for hours. These have not occurred previously. She feels that her lifestyle is severely compromised and is becoming depressed. There are no abnormalities on examination. Blood pressure is 140/80 mmHg. What is the most likely diagnosis? Migraine without aura Parotid tumour Cluster headaches Your answer Paroxysmal hemicrania Analgesic overuse headaches

The symptoms are characteristic of cluster headache. Migraine is possible, but the length of attack, the frequency and the occurrence first thing in the morning are all suggestive of cluster headache. Paroxysmal hemicrania causes pain of much shorter duration. There is no history of analgesic overuse. The possibility of structural abnormalities in the head or neck with this condition should always be considered, but the non-progressive nature makes it less likely in this case.

54. A 32-year-old intravenous drug abuser is admitted with a one-day history of double vision, droopy eyelids and a dry throat. Over the next 24 hours, her symptoms worsen and she begins to notice limb weakness and breathing difficulties. Examination reveals poorly reactive pupils, decreased deep-tendon reflexes and symmetrical upper and lower limb weakness, but sensation is normal. Cerebrospinal fluid examination is normal. The edrophonium test is positive. Electromyography shows no postactivation exhaustion. What is the most likely diagnosis? Botulism LambertEaton syndrome MillerFisher variant of the GuillainBarr syndrome Myasthenia gravis Poliomyelitis Your answer

Botulism is caused by a toxin produced by the anaerobic bacterium Clostridium botulinum, which blocks the release of acetylcholine from the motor nerve terminal. Botulism can be spread by the ingestion of foods contaminated by inadequate sterilisation, or, as in this case, through wound contamination in drug abusers after subcutaneous injection of heroin (the poor venous access of chronic addicts precludes intravenous heroin administration). Early symptoms are blurred vision, dysphagia and dysarthria. Pupillary responses to light are impaired, tendon reflexes reduced and typically there is progressive symmetrical limb weakness. Most patients have evidence of autonomic dysfunction such as dry mouth, constipation or urinary retention. The edrophonium test is falsely positive in about one-third of cases and does not distinguish botulism from other causes of neuromuscular blockade. Lambert Eaton Myasthenic syndrome(LEMS) could conceivably present with similar signs (reduced reflexes, autonomic signs), but the clinical context is less suggestive of this diagnosis, as LEMS is most often a paraneoplastic disorder; in addition the rapid decline would be very atypical of LEMS. Cerebrospinal fluid examination is associated with a high protein

content in GuillainBarr syndrome and with pleocytosis in poliomyelitis. In myasthenia gravis, there is postactivation exhaustion on electromyography.

55. A 28-year-old woman began to stumble when walking. Her right leg was slightly stiff and weak, especially after exercise and hot showers. These symptoms developed over 3 days and gradually disappeared over 4 weeks. She was on the college swimming team before these symptoms arose. There, she developed a unique and extreme type of fatigue that was different from the usual fatigue she experienced when swimming. This disappeared after several weeks, but had reappeared again 6 months before her current presentation. Which of the following diagnoses is most likely? Myalgic encephalomyelitis (chronic fatigue syndrome) Acute disseminated encephalomyelitis Multiple sclerosis Your answer GuillainBarr syndrome Diabetic sensorimotor neuropathy

Patients with multiple sclerosis commonly complain that their symptoms are worse with exercise and heat. They also experience profound fatigue. The focal neurological syndrome described in the initial paragraph is inconsistent with chronic fatigue syndrome. Acute disseminated encephalomyelitis tends to cause a more acute demyelinating syndrome with impaired consciousness. Guillain Barr syndrome and diabetic neuropathy are symmetrical.

56. In a patient with diplopia which one of the following findings is MOST suggestive of myasthenia gravis? Loss of pin prick sensation around the chin area

Preserved pupillary light reflex with absent accommodation reflex Thymoma on computed tomography scan (CT scan) of the chest Your answer Elevated creatinine phosphokinase (CPK) Proptosis

Myasthenia gravis is an acquired autoimmune disorder associated with acetylcholine receptor deficiency at the motor endplate. The ocular muscle involvement is usually, bilateral, asymmetrical and typically associated with ptosis and diplopia. Pupillary and accommodation reflexes are characteristically normal. Two-thirds of patients with myasthenia gravis have thymic hyperplasia and 10%15% will have thymoma. The creatinine phosphokinase (CPK) is typically normal. Exophthalmos and diplopia are suggestive of Graves disease. There is restriction in upward and/or outward gaze; this is due not to weakness of the superior eye muscles but to swelling and fibrosis of the inferior rectus and inferior oblique muscles beneath the globe.

57. A 25-year-old man presents with a 3-day history of diplopia and unsteadiness. He had had an upper respiratory infection 3 weeks ago. On examination there is bilateral partial ptosis, complete ophthalmoplegia, areflexia and gait ataxia. Sensory examination is normal, and plantar response flexor bilaterally. Given the likeliest clinical diagnosis, what is the most appropriate treatment? Azathioprine Immunoglobulins Prednisolone Pyridostigmine Thiamine

Your answer

The most likely diagnosis is Miller, Fisher syndrome, a GuillainBarr syndrome variant. It presents with a triad of ophthalmoplegia, areflexia and ataxia. It usually follows gastroenteritis or an upper respiratory tract infection. Positive anti-GQ1b ganglioside antibodies (serum and CSF) are diagnostic. The treatment of choice is either

immunoglobulins or plasma exchange. The majority of patients improve, but recovery may be slow (weeks to months).

58. A 48-year-old man, with a history of chronic intravenous drug abuse, presents with a severe subacute neuropathy. Nerve conduction studies show evidence of a multifocal axonal neuropathy. He is also noted to have nailfold infarcts in his hands and feet and hepatomegaly. Which of the following diagnoses is the most likely? Cytomegalovirus polyradiculoneuropathy Systemic toxoplasmosis Hepatitis B-related GuillainBarr syndrome Hepatitis C-related GuillainBarr syndrome Hepatitis C-related cryoglobulinaemia

Your answer

The presence of nailfold infarcts and the multifocal nature of the neuropathy indicate that a vasculitic cause is most likely. Hepatitis C infection may be associated with cryoglobulinaemia, which causes a vasculitic syndrome including neuropathy. Treatment of the vasculitis may include one or several of the following: high-dose intravenous steroids, plasma exchange, intravenous immunoglobulins. In addition, interferon therapy may be indicated for clearance of the hepatitis viral load. The latter is usually decided in conjunction with gastroenterology colleagues. Other conditions associated with vasculitic neuropathy include polyarteritis nodosa, ChurgStrauss syndrome, rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and Wegeners granulomatosis.

59. A 42-year-old man has been in the intensive care unit for 6 weeks following a road traffic accident in which he had crashed his car after drinking heavily. You are called to see him as the staff has noticed that he appears to have developed some distal limb weakness over the past 710 days. On examination a symmetrical distal weakness is confirmed and he appears to have absent ankle and knee reflexes. There is some wasting of the intrinsic hand muscles bilaterally, worse on the right. He has possible patchy sensory loss in a glove and stocking distribution, although this is inconsistent on testing. Which of the following is the most likely diagnosis? GuillainBarr syndrome Critical illness polyneuropathy

Your answer

Critical illness myopathy Nutritional polyneuropathy Alcoholic polyneuropathy Prolonged periods in intensive care, irrespective of the underlying pathology, are associated with a risk of developing critical illness polyneuropathy. This is an axonal neuropathy and thus muscle wasting may occur. It may be predominantly sensory, predominantly motor or mixed. Nerve conduction studies and electromyography may be indicated to confirm the diagnosis and exclude any other condition that may require specific therapy (eg GuillainBarr syndrome). A patient with a previous history of chronic alcohol misuse, diabetes or another condition predisposing to neuropathy may be more prone to develop significant critical illness polyneuropathy.

60. A 30-year-old man is brought to the emergency department with severe confusion and ataxia. On examination he has bilateral VIth nerve palsies and gait ataxia. He is disoriented in time and place. A full blood count shows an MCV of 110 fl. CT brain and EEG are both normal. What immediate treatment should be administered? Intravenous aciclovir Intravenous cefuroxime Intravenous dexamethasone Intravenous diazepam Intravenous thiamine

Your answer

The combination of confusion, ataxia and ophthalmoplegia are the typical features of Wernickes encephalopathy. Other less common features include autonomic dysfunction and hypothermia. The high MCV is most likely to be consistent with chronic alcohol abuse. Other causes of Wernickes encephalopathy include vomiting and chemotherapy. Immediate intravenous thiamine is the treatment of choice.

61. A 69-year-old woman presents with a sudden onset of weakness of her right arm and leg. She is known to be hypertensive. There has been no headache, loss of consciousness, visual, speech or sensory symptoms. On examination, her blood pressure is 180/90 mmHg, pulse 100 and regular. Heart sounds are normal. There is no carotid bruit. Higher mental function tests are normal. There is no apraxia or neglect. Speech, swallowing and sensation are all normal. There is no visual field defect. There is a mild facial weakness sparing the forehead. The right arm and leg are flaccid and weak. Reflexes and tone are normal. There is a right extensor plantar response. What is the most likely cause of this patients symptoms?

Cardioembolic stroke Lacunar stroke Right internal carotid artery atheroembolic stroke Right internal carotid artery dissection Right vertebral artery atheroembolic stroke

Your answer

A sudden onset of focal symptoms and signs strongly suggests stroke. The presence of right-sided weakness involving the face, arm and leg, in the absence of sensory loss, language disturbance, hemineglect or visual field defect favours a small deep (lacunar) infarct affecting the corticospinal tract at the level of either the internal capsule or the upper pons. The sparing of the forehead, indicating upper motor-neurone facial weakness, favours the former diagnosis. The term lacune refers to a small deep infarct resulting from occlusion of a penetrating branch. They occur predominantly in the basal ganglia, internal capsule, thalamus and pons. Hypertension and diabetes are the main risk factors. Common lacunar strokes include a pure motor hemiparesis, pure hemisensory loss, ataxic hemiparesis and dysarthria clumsy-hand syndrome.

62. A 55-year-old woman presents with a four year history of memory loss and difficulty speaking. She has also been noted to be abulic and apathetic. On examination she has difficulty in naming some common objects and is also noted to have some positive grasp and palmomental reflexes. What is the most likely diagnosis? Picks disease Corticobasal degeneration Alzheimers disease Wilsons disease CreutzfeldtJakob disease Your answer

Pick's disease is a type of dementia characterised by a slowly progressive deterioration of social skills and changes in personality, along with impairment of intellect, memory, and language. It tends to affect individuals at a younger age than Alzheimer's: peak incidence occurs at 55-65 years. Symptoms may include loss of memory, lack of spontaneity, difficulty in thinking or concentrating, and disturbances of speech, gradual emotional dullness, loss of moral judgement and progressive dementia. Speech and language dysfunction may occur early and progress rapdily. These neuropsychological symptoms reflect the pathology of the disease, which typically affects frontal and temporal lobes causing severe atrophy. Microscopically some nerve cells have characteristic abnormalities (Pick bodies). Although the disease usually affects individuals between the ages of 40 and 60, the age of onset may range from 20 to 80.

63. An 18-year-old girl presented at the age of 3 years with progressive ataxia. She is now wheelchair-bound. On examination, she is now dysarthric, with bilateral optic atrophy. There is ataxia in both upper limbs. Reflexes in her lower limbs are absent with bilateral extensor plantar response. She has absent vibration and impaired joint position in both feet. Bilateral pes cavus is apparent. ECG shows inverted T waves. Echocardiogram reveals left ventricular hypertrophy. What is the most likely diagnosis? CharcotMarieTooth disease Friedreichs ataxia Your answer Huntingtons disease Multiple sclerosis Vitamin B12 deficiency

Friedreichs ataxia is considered the most common hereditary ataxia. It is an autosomal-recessive disorder with variable phenotype. Onset is usually in early life and life expectancy is generally about 35 years. Associated features include ataxia, dysarthria, optic atrophy, hearing impairment, learning difficulties, sensory neuropathy, extensor plantar responses, diabetes and cardiomyopathy. Pes cavus and kyphoscoliosis are also common features. Friedreichs ataxia is one of the causes of absent reflexes in the lower limbs and extensor plantar responses.

64. A 27-year-old man presents with a 2-day history of drooling when he tries to eat, difficulty closing his eyes and inability to smile. On examination he has weakness of eye closure, which is worse on the left, and weakness of other muscles of facial expression. Facial sensation is normal, as are eye movements, hearing and bulbar function. The remainder of both the neurological and general examination is likewise normal. There is a family history of stroke. His previous health has been good with no recent illness. What is the most likely diagnosis? Basilar artery territory stroke

Myasthenia gravis Sarcoidosis Lyme disease Bilateral Bells palsy Your answer The clinical signs are those of bilateral lower motor neurone seventh (facial) nerve palsy lower motor neurone as eye closure is impaired. There are a few causes of this including sarcoidosis, Lyme disease and bilateral parotid infiltration (eg by a lymphoma). However, in this case, with no preceding illness and no associated signs, the cause is most likely to be Bells palsy, which can occasionally present bilaterally. The aetiology of this is unclear but some cases may have a viral origin. The value of steroid therapy and/or antiviral therapy for this condition is unclear and trials are underway in the UK. This patient will need careful eye care to avoid corneal abrasion and infection.

65. An elderly man presented with progressive lower limb weakness, particularly with hip flexion, arreflexia of bilateral knees and ankles with bilateral extensor plantars. He had a history of prostate carcinoma. Investigations : raised alkaline phosphatase (ALP), hypercalcaemia, normal parathyroid hormone (PTH). What is the next most appropriate investigation? Magnetic resonance imaging (MRI) of the spine Computerised tomography (CT) of spine Bone density scan Myelogram Plain films of the spine Your answer

This patient has clear evidence of progressive spinal cord compression. Extradural tumours principally involved in causing spinal cord compression include carcinomas of the bronchus, breast, prostate and thyroid, lymphoma and malignant melanoma. Plain films may show degeneration of vertebrae and destruction, but MRI is the best investigation for

identifying the size and nature of any destructive lesion. Acute management involves use of corticosteroids to reduce oedema, followed by referral for local radiotherapy.

66. A 62-year-old retired postman awoke with darkened, impaired vision in the upper half of the visual field of his left eye 3 days prior to evaluation. He described the onset of symptoms as like a shade being pulled down over the visual picture. He did not complain of eye pain. He had noted headache for 6 weeks beforehand, and had consulted his GP on three occasions about this. He was told that he was suffering from tension headache, and was prescribed simple analgesics. When questioned specifically he reports having pain at each side of his jaw when he eats, especially towards the end of the meal. He is hypertensive and on treatment with a -blocker. He has never smoked. Which of the following diagnoses is likely? Central retinal artery occlusion Non-arteritic ischaemic optic neuropathy Migraine Arteritic ischaemic optic neuropathy Your answer Optic neuritis

Altitudinal defects (from the bottom up, or the top down) are common in vascular disorders of the optic nerve head. Vascular problems are common on waking. The preceding headache raises the suspicion of temporal arteritis, the feared complication of which is arteritic anterior ischaemic optic neuropathy. Jaw claudication as described above is pathognomonic of temporal arteritis. Non-arteritic ischaemic optic neuropathy is common in patients with multiple vascular risk factors, does not cause headache and is not associated with raised inflammatory markers. Neurological signs precede headache in migraine, which would be uncommon in this age group, as would optic neuritis.

67. A 56-year-old man is referred to you with a 2-year history of ascending lower limb numbness and, more recently, foot drop. In the last 6 months he has also developed numbness in his fingers. He has a distal reduction to pinprick, relatively preserved muscle power, except for ankle dorsiflexion and hyporeflexia in his legs. The GP has already organised nerve conduction studies and the report is sent along with the patient. Which of the following is suggestive of an axonal neuropathy? Reduced conduction velocity Abnormally slow F-response Delayed P100 latency Reduced compound muscle action-potential amplitude Your answer Conduction block Reduced conduction velocity is associated with demyelinating neuropathies. An abnormally slow F-response is associated with very proximal disease, ie radiculopathies. Delayed P100 latency is a feature of performing visual evoked potentials in those with optic nerve disease. Conduction block is usually associated with certain types of demyelinating neuropathy.

68. A 40-year-old banker came to the emergency room complaining of intense headache of two hours duration. The pain is localised around the right eye and is associated with tearing and redness of the eye. The patient reported he has had similar episodes over the last year. He also admits that these episodes occurred every day for a few weeks with one to three attacks a day which last for one to two hours, frequently at night. After six weeks, the attacks stopped. He lost his job six months ago and has noticed an increase in the intensity of the pain since. Examination reveals drooping of the eyelid and small pupil on the right side. Which one of the following is the MOST probable diagnosis? Migraine Tension-type headache

Iritis associated headache Cerebral tumour Cluster headache Your answer The features are classical for cluster headache. Cluster headache afflicts less than one in a thousand in the general population. The majority of sufferers are men. The syndrome is characterised by frequent attacks of intense pain localised in and around the eye on one side, characteristically accompanied by conjunctival injection and lacrimation in this eye, along with nasal stuffiness on the same side and sometimes Horners syndrome. All signs and symptoms are strictly unilateral and occur during attacks lasting between 15 minutes and three hours. The attacks occur from one to eight daily during a period lasting from some weeks to months. After a remission of varying duration, the same pattern recurs. In contrast to migraine, during an attack the cluster patient prefers to pace about. Attacks frequently occur at night. Recent findings suggest a pivotal role of the hypothalamus in relation to the pathophysiology. Sumatriptan injection or oxygen inhalation aborts pain attacks in most patients. The most frequently used prophylactic agents are verapamil, lithium and steroids.

69. A 28-year-old woman was diagnosed 3 months ago with schizophrenia. Her psychiatrist started her on haloperidol. She has now presented to A&E with confusion and drowsiness. On examination she is pyrexial (39.5 C), disoriented and her blood pressure is 200/100 mmHg. She has severe bilateral rigidity. What treatment should be administered? Aciclovir Cefuroxime Dantrolene Nifedipine Propranolol

Your answer

The most likely diagnosis is neuroleptic malignant syndrome. It is a known complication of neuroleptic treatment. It presents with an altered mental state, pyrexial, rigidity and autonomic dysfunction. Management consists of muscle relaxants, such as dantrolene, and

intravenous fluids. Dopamine agonists need to be considered but may exacerbate the psychiatric condition.

70. A 50-year-old woman presents following a fall. She reports pain and weakness in her hands for several months, stiff legs and swallowing difficulties and has bilateral wasting of the small muscles of her hands. Reflexes in the upper limbs are absent. Tongue fasciculations are present and both legs show increased tone, pyramidal weakness and hyperreflexia with extensor plantars. Pain and temperature sensation is impaired in the upper limbs. What is the most likely diagnosis? Multiple sclerosis Motor neurone disease Syringobulbia Syringomyelia Cervical spondylosis

Your answer

This patient presents with lower motor neurone (LMN) signs in her arms and upper motor neurone (UMN) signs in her legs, indicating a lesion in the spinal cord. There is also a dissociated sensory loss of thermal and pain sensation sparing the posterior columns. This is typical of a syrinx. The LMN lesion of the XIIth nerve proves that the lesion extends into the brainstem, ie syringobulbia.

71. A 75-year-old woman had a left total hip replacement yesterday. Today she has developed weakness and numbness of her left foot. On examination there is weakness of all ankle movements (dorsiflexion, plantarflexion, eversion and inversion) and numbness over the dorsum of her foot and lateral aspect of her leg. The left ankle jerk is absent. Where is the most likely site of the lesion? Left common peroneal nerve Left femoral nerve Left obturator nerve Left sciatic nerve Left tibial nerve

Your answer

Sciatic nerve damage is a common complication of total hip replacement. It causes global weakness of the ankle due to the involvement of both its branches (common peroneal and tibial nerve). The ankle jerk is absent due to the involvement of the tibial nerve. Sensory

loss is variable but most commonly occurs around the dorsum of the foot and lateral aspect of the leg. Common peroneal nerve palsy causes weakness of ankle dorsiflexion and eversion. Tibial nerve palsy causes weakness of ankle plantarflexion and inversion. Femoral nerve palsy causes weakness of hip flexion and knee extension. Obturator nerve palsy causes weakness of hip adduction.

72. A 30-year-old pregnant woman presents with malaise, headache, diarrhoea and neck stiffness. On examination she has a temperature of 38.2 C and mild meningism. Her general practitioner had prescribed oral antibiotics earlier that day. After a CT brain scan, which was normal, CSF examination shows a slightly elevated protein of 0.6 g/dl, a white cell count of 200/mm3 (mostly lymphocytes), CSF glucose level of 3.3 mmol/l and blood glucose of 4 mmol/l. Which of the following is the most likely diagnosis? Viral meningitis due to echovirus Partially treated streptococcal meningitis Listeria meningitis Cryptococcal meningitis Tuberculous meningitis Your answer

The pattern of slightly elevated protein, lymphocytosis and normal CSF/blood glucose ratio is most suggestive of viral meningitis. The other aetiologies typically cause a low (bacterial) or very low (tuberculous, fungal) CSF/blood glucose ratio. Partial treatment of bacterial meningitis may give a lymphocytic rather than a polymorph infiltrate, but the glucose ratio still tends to be low. Listeria meningitis is more common in pregnant women.

73. A 30-year-old pregnant woman presents with malaise, headache, diarrhoea and neck stiffness. On examination she has a temperature of 38.2 C and mild meningism. Her general practitioner had prescribed oral antibiotics earlier that day. After a CT brain scan, which was normal, CSF examination shows a slightly elevated protein of 0.6 g/dl, a white cell count of 200/mm3 (mostly lymphocytes), CSF glucose level of 3.3 mmol/l and blood glucose of 4 mmol/l. Which of the following is the most likely diagnosis? Viral meningitis due to echovirus Partially treated streptococcal meningitis Listeria meningitis Cryptococcal meningitis Tuberculous meningitis Your answer

The pattern of slightly elevated protein, lymphocytosis and normal CSF/blood glucose ratio is most suggestive of viral meningitis. The other aetiologies typically cause a low (bacterial) or very low (tuberculous, fungal) CSF/blood glucose ratio. Partial treatment of bacterial meningitis may give a lymphocytic rather than a polymorph infiltrate, but the glucose ratio still tends to be low. Listeria meningitis is more common in pregnant women.

74. Which one of the following is MOST suggestive of a lesion of the sciatic nerve? Absent knee tendon jerk Foot drop Your answer Inability to flex the hip Decreased sensation on anterior thigh and medial leg Intervertebral disc prolapse at L2/L3 levels

The sciatic nerve originates in the sacral plexus mainly from the spinal segment L5 to S2. It supplies muscles that cause extension of the thigh and flexion of the leg. It divides into two major branches, the tibial nerve and the common peroneal nerve, which are responsible for all foot movements. Anterior thigh and medial leg sensory loss is typical of femoral nerve lesion. The femoral nerve mediates flexion of the hip. Sciatica is pain in the distribution of the sciatic nerve regardless of the aetiology.

75. A 37-year-old woman is referred to your clinic with two episodes of suddenonset limb paralysis at night-time over the last 4 months. She describes waking shortly after falling asleep and being aware of an inability to move her limbs or to speak, associated with a feeling of suffocation lasting for about a minute. Her father recently died from an intracerebral haemorrhage during sleep. She has no other symptoms other than daytime sleepiness, which she puts down to looking after her two young children. There are no abnormalities on examination. What is the most likely diagnosis?

Depression and anxiety Nocturnal seizures Brainstem transient ischaemic attack Narcolepsy Your answer Cervical disc prolapse This woman is describing sleep paralysis. It reflects the atonia of REM sleep. Awareness is preserved during the attack, which heightens the sense of terror and anxiety, especially if a feeling of suffocation is present (although the respiratory muscles are only ever mildly affected in comparison to the limbs). Sleep paralysis may be isolated or occur in the context of narcolepsy, in which it affects 1545% of patients.

76. Interferon is a licensed treatment in the United Kingdom for which of the following neurological conditions? First episode of optic neuritis Relapsing remitting multiple sclerosis Primary progressive multiple sclerosis Motor neurone disease GuillainBarr syndrome

Your answer

In the UK, -interferon is currently a licensed disease-modifying therapy for relapsing remitting multiple sclerosis (MS) and secondary progressive MS in adults. Clinical trials have shown a modest reduction in relapse frequency and a reduction in MRI lesion load, but no impact on the final level of disability. Relapsing remitting disease, for the purposes of interferon eligibility, is defined as two or more clinically significant relapses in the 2 years preceding prescription. The patient must be able to walk, with or without a walking aid, a distance of at least 10 metres. The treatment is given as either subcutaneous injection three times per week or intramuscular injection once weekly. Adverse effects include local inflammatory reactions at injection sites, flu-like symptoms and depression. Some patients develop autoantibodies to interferon that limit its efficacy. At present, treatment with interferon is not recommended for clinically isolated syndromes (such as optic neuritis) or primary progressive MS, due to lack of evidence.

77. Which of the following is a good first-choice oral treatment for primary generalised epilepsy in a 20-year-old man? Topiramate Valproate Your answer Carbamazepine Phenytoin Phenobarbital

There is insufficient evidence to support the use of first-line treatment with topiramate for any seizure type. Nowadays, phenobarbital should not be initiated for epilepsy treatment due to its unacceptable side-effects when compared with more modern alternatives. Phenytoin is a useful drug acutely, but again has troublesome problems associated with longer term use, and would generally be avoided in this age group. The first-choice treatments for primary generalised epilepsy are lamotrigine and valproate. Carbamazepine, valproate, lamotrigine and oxcarbazepine are all reasonable first choices for partial (localisation-related) epilepsy.

78. A 54-year-old man presents with slowly progressive facial weakness and loss of taste. He has also noticed that he is more sensitive to loud sounds than usual. On examination there is weakness of the facial muscles on the right (including the forehead), a small patch of altered sensation on the right cheek, and decreased taste sensation. Where is the most likely location of this lesion? Cerebellopontine angle Parotid gland Internal auditory canal Petrous temporal bone Your answer

Stylomastoid foramen The facial nerve emerges from the brainstem with the nervus intermedius, which gains its name from its position as it courses across the cerebellopontine angle (CPA) between the facial nerve and the vestibulocochlear nerves (ie cranial nerves VII and VIII). Cranial nerve V (trigeminal) also lies in the cerebellopontine angle. The close anatomical association between these structures at the level of the cerebellopontine angle may result in disturbances in tearing, taste, salivary gland flow, hearing, balance and facial sensation and facial weakness as the result of lesions at this level.

79. A 65-year-old man presents with sudden-onset visual disturbance. He denies any other symptoms. He is known to be a heavy smoker and hypertensive. On examination he has a right incongruous homonymous hemianopia. Where is the most likely site of the lesion? Left occipital lobe Left optic nerve Left optic tract Left temporal lobe Optic chiasma

Your answer

Incongruous homonymous hemianopia is a feature of an optic tract lesion. An optic chiasm lesion causes bitemporal hemianopia. A temporal lobe lesion causes a superior homonymous quadrantanopia. An occipital lobe lesion causes a congruous homonymous hemianopia. An optic nerve lesion causes a scotoma.

80. A 40-year-old man describes intermittent unilateral pain above and behind his left eye, which has woken him from sleep every night for the previous 7 days. The pain is described as severe and stabbing, lasting about 30 minutes and making him restless and agitated. It is associated with tearing from his left eye and nasal stuffiness. When he looked in the mirror during attacks he had noted his left eyelid drooping. He remembers he had experienced similar symptoms for about a month last year, but that they had resolved spontaneously and he had not sought medical attention. Physical examination is normal. He is a lifelong smoker. What is the diagnosis? Migraine Cluster headache Your answer

Temporal arteritis Paroxysmal hemicrania Trigeminal neuralgia The symptoms are very suggestive of cluster headache, the most common of a group of headache disorders known as the trigeminal autonomic cephalgias. This commonly occurs in middle-aged men, and occurs classically in clusters lasting weeks to months. Paroxysmal hemicrania is another type of trigeminal autonomic cephalgia, but is much rarer, tends to occur multiple times throughout the day and is commoner in women. Trigeminal neuralgia is sometimes mistaken for cluster headache, but the attacks are much shorter and autonomic activation is rare. Sometimes cluster headache can have migrainous features, but migraine lacks the specific features stated in the question. Temporal arteritis would be unheard of in this age group. Verapamil was found to be more effective than placebo in a randomised controlled trial of treatment for cluster headache. Prednisolone is also sometimes used for treatment, but its use is not supported by randomised evidence.

81. A 30-year-old pregnant woman presents with a weak grip and tingling of her right hand. She complains of a dull aching pain in her forearm, which is made worse by carrying a shopping bag. On examination you find weakness of the right abductor pollicis brevis and mild weakness of thumb flexion. Finger abduction and adduction appear to be within normal limits. There is sensory loss to pinprick mainly affecting the right thumb and index finger. Phalens sign is positive. The left-hand sensorimotor examination is normal. The deep tendon reflexes are symmetrical. What is the most likely cause of her symptoms? Compression of the right ulnar nerve at the elbow Right C8 nerve root irritation Right brachial plexopathy

Compression of the right median nerve in the forearm Compression of the right median nerve in the carpal tunnel Your answer Her symptoms suggest carpal tunnel syndrome. Pregnancy is a risk factor for this, as are other conditions that promote fluid retention or thickening of the subcutaneous tissues (eg hypothyroidism), factors that alter the configuration of the wrist structures (eg osteoarthritis or rheumatoid arthritis) and conditions that predispose to neuropathy (eg diabetes, hereditary tendency to pressure palsy). Although the median nerve supplies the lateral two lumbricals, opponens pollicis, abductor pollicis brevis and flexor pollicis longus (mnemonic LOAF), the pattern of weakness may be incomplete. This also applies to the pattern of sensory loss. Phalens sign involves placing pressure over the carpal tunnel with the wrist flexed it is considered positive if this reproduces the patients symptoms (although, of course, it is neither 100% specific nor sensitive). Nerve conduction studies would confirm the diagnosis.

82. A 19-year-old college student is noted to seem confused by her flatmate. She has been complaining of a diffuse headache and general malaise for the past 24 hours. On examination she has a temperature of 38 C. She is restless and mildly dysphasic. The remainder of the general and neurological examination is normal. CT brain scan shows hypodensity in both temporal lobes. Cerebrospinal fluid (CSF) examination shows a white cell count of 16/mm3 (lymphocytes), a slightly raised protein concentration of 0.75 g/l and a normal CSF/blood glucose ratio. Which would be the most appropriate early management pending further diagnostic information? Intravenous fluids, broad-spectrum antibiotics and prophylactic anticonvulsants pending further CSF analysis Intravenous fluids, aciclovir and prophylactic anticonvulsants Intravenous fluids, aciclovir and broad-spectrum antibiotics

Intravenous fluids, aciclovir and corticosteroids Intravenous fluids and iv aciclovir

Your answer

The short history and presence of dysphasia are both characteristic of herpes simplex encephalitis (HSE), which is caused by herpes simplex virus (HSV): HSV-1 in 90% of cases, and in the remainder HSV-2, mainly neonates and immunocompromised patients. The presence of temporal lobe changes (focal oedema) on neuroimaging is typical of HSV (though note that CT may be normal in the early stages MRI is much more sensitive). Diagnosis may be confirmed by CSF polymerase chain reaction (PCR) for HSV (specificity > 95%; sensitivity should be 95% in an experienced laboratory; though there may be false-negatives very early or very late). Infective encephalitis must be distinguished from other causes of encephalopathy (eg metabolic, ischaemic, toxic, etc). Many other viruses cause encephalitis (varicella, EpsteinBarr virus (EBV), influenza, etc). However, HSE is the most important to consider because of the existence of an effective antiviral agent; aciclovir treatment should always be given in adequate doses for any possible case, and continued for a full course of at least 10 days unless an alternative diagnosis is found. Steroids may be given if there are signs of significant cerebral swelling, although the evidence base is limited. Anticonvulsants are used to treat symptomatic focal and generalised seizures, though are not routinely given prophylactically. This patient has a good prognosis based on her young age, short duration of illness and absence of coma.

83. A patient with a history of left optic neuritis 1 year ago, and transient spastic paraplegia (lasting 6 weeks) 6 months ago is seen in clinic. His T2 -weighted MRI scan shows multiple periventricular hyperintensities and three similar lesions in the posterior fossa, with one lesion in the corpus callosum. Lumbar puncture shows oligoclonal bands. Only a relative afferent pupillary defect on the left is evident on examination. So far, he has not been treated for his problems. What treatment should he be offered? Plasmapheresis Intravenous methylprednisolone

Oral prednisolone -Interferon Azathioprine This patient has multiple sclerosis as evidenced by 2 spearted episodes of neurological dysfunction, a typical MRI appearance and characteristic immunological changes in the CSF. This patient fulfils the currently accepted UK criteria for the prescription of -interferon. Randomised evidence now exists that interferon given after the first attack reduces the likelihood of a second attack within 2 years, and numerous randomised trials have shown a reduction of the relapse rate (by a one-third to one-half) in patients with definite multiple sclerosis (like this patient). Evidence on the use of azathioprine in this situation is intriguing, but it is not accepted as standard therapy. Oral or iv methylprednisolone shortens the relapse duration, but is not used to prevent relapse. Oral prednisolone has no role in MS treatment. Plasmapheresis is used as a rescue therapy if steroids fail to help in cases of severe relapse.

Your answer

84. A 28-year-old man presents to the A&E department complaining of a suddenonset occipital headache associated with vomiting. His symptoms started 2 hours previously and are continuing. He has a previous history of infrequent migraine without aura, which also causes nausea but not vomiting. His current headache is much more severe than his usual migraine, which has not previously started suddenly. Examination is unremarkable. In particular there is no neck stiffness or photophobia. Which of the following management options would be the most appropriate? A diagnosis of migraine and suggestion that he be referred to a neurologist for a further opinion Immediate lumbar puncture, to exclude meningitis and subarachnoid haemorrhage CT brain scan, followed by discharge if normal CT brain scan, followed by lumbar puncture if CT normal

Your answer

CT brain scan, followed by angiography

It is certainly likely that this represents recurrent migraine, but there are concerning features that make this a diagnosis of exclusion. The sudden onset and greater severity than his usual migraine requires that subarachnoid haemorrhage be excluded. CT brain scan is normal in up to 30% of patients with subarachnoid haemorrhage. However, a positive CT brain scan is diagnostic. If the CT is negative for blood, a lumbar puncture is needed to look for red blood cells. The absence of red blood cells or of xanthochromia excludes subarachnoid haemorrhage. Because bilirubin and oxyhaemoglobin take 12 hours to appear in the cerebrospinal fluid following a bleed, lumbar puncture should usually be delayed until more than 12 hours from the onset of symptoms.

85. A 60-year-old man with chronic atrial fibrillation is on warfarin. He has had two unprovoked tonicclonic seizures that were witnessed by his wife. CT brain shows only some diffuse ischaemic changes in the white matter. The GP wants to start him on an antiepileptic drug. Which of the following antiepileptic drugs does not have important drug interactions with warfarin? Carbamazepine Lamotrigine Phenytoin Phenobarbital Primidone

Your answer

Drug interactions should always be considered before introducing antiepileptic drugs. Phenytoin, carbamazepine, primidone and phenobarbital are liver enzyme inducers. Sodium valproate is a liver enzyme inhibitor. Lamotrigine has no effect on liver enzymes and is the treatment of choice for the above patient.

86. A 26-year-old woman presents to her GP with mood swings and depression. She is prescribed Prozac, but returns a few months later with pains and numbness in both legs. Her antidepressant is changed but her mood worsens and she starts to have problems with her memory and motivation. She is referred to your clinic where, in addition to mild cognitive impairment, you notice slight increased tone in her limbs and occasional myoclonic jerks. Her MRI scan reveals thalamic hyperintensity on T2-weighted images, but her EEG is normal. What is the most likely diagnosis?

Corticobasal degeneration Progressive severe depression Juvenile myoclonic epilepsy New-variant CreutzfeldtJakob disease Wilsons disease

Your answer

This patient is too young to develop corticobasal degeneration. Continuing depression is also unlikely in view of the abnormal neurological signs. She does have myoclonus but not seizures. Wilsons disease may present with psychiatric symptoms, as well as disorders of movement, particularly tremor, but new-variant CreutzfeldtJakob disease is more likely. New-variant CJD commonly presents with psychiatric symptoms in young adults, followed by non-specific painful sensory symptoms, most often in the lower limbs. Cognitive impairment, pyramidal signs, myoclonus and primitive reflexes may then develop. Mean disease duration is approximately 14 months. MRI commonly shows a high signal on T2weighted images in the pulvinar (posterior aspect of thalamus). EEG is often normal, compared to sporadic CJD in which triphasic waves are observed. Diagnosis is made antemortem by brain biopsy or, more commonly, by tonsillar biopsy.

87. A 30-year-old patient presents with sudden monocular visual loss on the right associated with pain behind the eye and alteration of colour vision. Examination the following day reveals a relative afferent pupillary defect in the right eye. Which of the following diagnoses is most likely? Cerebral infarction Optic neuritis Your answer Optic nerve glioma Migraine Temporal arteritis The history is suggestive of an acute optic nerve lesion, the most likely diagnosis in this age group being optic neuritis. Hemianopic visual disturbance is the common type seen in cerebral infarction. Optic nerve glioma usually presents over a longer period, often with proptosis. Migraine can cause monocular

disturbances, but these are usually in the context of headache, and are transient. Temporal arteritis does not occur in patients < 50 years of age.

88. You are referred a 57-year-old woman with a marked change in personality over the last 5 years. She has become increasingly sexually flirtatious with inappropriate behaviour in social situations. Impairment of abstract thinking, memory and planning has become increasingly obvious. However, the ability to perform arithmetic tasks is relatively preserved. Speech output is diminished. There is no motor impairment. Physical examination is unremarkable except for the presence of grasp reflexes. What is the most likely diagnosis? Normal-pressure hydrocephalus Frontotemporal dementia Your answer Alzheimers disease Frontal lobe meningioma Multiple sclerosis

The prominent early feature of Alzheimers disease is short-term memory impairment, which is not really a feature of this womans presentation. Frontotemporal dementia, however, is associated with early personality change, although the exact nature of this change varies. Patients may present with disinhibition, or alternatively with apathy, loss of initiative and drive with impairment of frontal executive tasks. Speech gradually deteriorates and many patients develop aphasia. Motor skills are well preserved. Neuroimaging might reveal isolated frontotemporal lobar atrophy. Normal-pressure hydrocephalus would be likely to have caused gait disturbance by now. Multiple sclerosis can cause frontal signs, but is likely to have presented with visual or spinal symptoms. A frontal lobe meningioma might be associated with features of raised intracranial pressure, and possibly anosmia.

89. A 69-year-old woman has become progressively weaker over a 3-month period. Her symptoms fluctuate diurnally, being worse when she awakes. Although her greatest difficulty is climbing stairs, she also has difficulty raising her arms. The patient denies the presence of pain, numbness, diplopia, difficulty swallowing and bladder abnormalities, but has noted dryness of her mouth. She has been a heavy smoker for years. On examination there are no abnormalities of the cranial nerve, cerebellar or sensory function. Her weakness is diffuse and mild, with strength increasing with exertion. Reflexes are absent but are obtainable after exertion. Plantar responses are flexor. What is the most likely diagnosis? LambertEaton myasthenic syndrome Motor neurone disease Multiple sclerosis Myasthenia gravis Polymyositis Your answer

LambertEaton myasthenic syndrome is an antibody-mediated syndrome (anti-voltagegated calcium antibodies) often associated with small-cell lung carcinoma or with autoimmune diseases. It starts insidiously, often several years before a tumour becomes evident. Patients present with proximal weakness, which is typically worse in the morning and decreases with exertion. There are accompanying autonomic features, especially dry mouth. It is a presynaptic neuromuscular disease with inhibition of acetylcholine release from the presynaptic junction. 90. A 13-year-old boy presents to A&E one morning after an episode of unconsciousness, a convincing history of which was obtained from his mother. She had witnessed his eyes rolling upwards, a fall to the floor, jerking of all four limbs and his lips going blue. He had been incontinent. He had been assessed in a general medical clinic 3 years earlier complaining of sudden brief bilateral jerks occurring largely in the morning after waking. Neurological examination is normal. What is the most likely diagnosis? Complex partial epilepsy Juvenile myoclonic epilepsy Your answer Hypoxic myoclonus Wilsons disease Pseudoseizures

This is a classic history for juvenile myoclonic epilepsy, one of the most common primary generalised epilepsy syndromes. Juvenile myoclonic epilepsy typically appears in the second decade. It is characterised by myoclonic seizures, associated at times with generalised tonicclonic seizures (as in this case) or absence seizures. The cardinal seizure type is that of myoclonic jerks characterised by sudden, brief, bilaterally symmetrical and synchronous muscle contractions. Consciousness remains unimpaired during myoclonic seizures, even if they occur in series or in myoclonic status epilepticus. Generalised tonicclonic seizures appear after the onset of myoclonic seizures in the majority of cases. Both the myoclonic seizures and generalised tonicclonic seizures have a special circadian pattern, in that they occur almost exclusively on or soon after awakening, either from all-night sleep or from a nap. The condition is treated with valproate or lamotrigine. Complex partial (localisation related) epilepsy is uncommon in this age group, and not usually associated with myoclonus. There is no history to suggest hypoxia, or the types of movement disorder commonly associated with Wilsons disease. This history does not sound non-organic.

91. Which of the following findings best supports a diagnosis of multiple sclerosis? CSF protein of > 1.2 g/dl Intrathecal synthesis of IgG Low CSF : serum glucose ratio Multiple grey matter lesions on MRI Normal visual evoked potentials

Your answer

Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. It most often affects young adults between the ages of 20 and 50, with a 2 : 1 female/male ratio. Cerebrospinal fluid is typically examined for the presence of increased intrathecal synthesis of IgG and also for the presence of a discrete set of proteins, called oligoclonal bands, that appear on gel electrophoresis. Note that oligoclonal bands and increased rates of IgG synthesis are found in about 90% of patients with definite multiple sclerosis, but may also be present in other diseases.

92. A 59-year-old man has shown a change in his mood and personality over a 9-month period. He has subsequently developed difficulty with memory and concentration, and then progressive fidgety movements of his limbs and facial musculature. By the time of

medical assessment he has frank choreiform movements and a Mini-Mental State Examination of 21/30. Other examination is normal. He was adopted and therefore no information on his family history is available. He has three adult children (aged 27, 30 and 33 years) who are asymptomatic. Based on the likely clinical diagnosis, which of the following genetic patterns of this condition is most likely? Autosomal-dominant inheritance with anticipation Autosomal-dominant with variable penetrance Autosomal-recessive inheritance X-linked inheritance Mitochondrial disorder Your answer

The clinical scenario is that of Huntingtons disease (also called Huntingtons chorea). This is characterised by slowly progressive subcortical dementia with early personality changes (irritability, disinhibition) followed by the onset of a progressive choreiform movement disorder. It has an autosomal-dominant mode of inheritance and is one of the trinucleotiderepeat disorders (CAG repeat, on the huntingtin gene, chromosome 4p16.3). Increasing repeat length in offspring gives rise to the phenomenon of anticipation (clinical onset at an earlier age in subsequent generations). Referral for genetic counselling prior to genetic testing is therefore an important step if the disease is suspected. Haloperidol and tetrabenazine can be used for symptomatic treatment of the chorea but the condition itself inexorably progresses, with terminal stages being reached after 1015 years in most patients.

93. A 78-year-old man presents with transient left arm weakness and collapse. He recovers within a period of 24 h and has a carotid ultrasound scan. This reveals a 55% stenosis affecting the right internal carotid artery and a 90% stenosis affecting the left internal carotid artery. Other risk factors of note include smoking of 30 cigarettes per day, which he refuses to stop and hypertension. Blood pressure in the clinic is 145/80 mmHg, he is in sinus rhythm and cholesterol is 5.1 mmol/l. Which of the following is the most appropriate management to reduce risk of a further stroke affecting the same territory? Aspirin therapy Aspirin and dipyridamole MR therapy Right carotid endarterectomy Left carotid endarterectomy Start statin therapy

Your answer

The right carotid stenosis is not severe enough to justify surgical intervention and the symptoms are not suggestive of left internal carotid artery stroke; carotid endarterectomy is

therefore excluded as an option. While aspirin alone reduces stroke risk, the combination of aspirin and dipyridamole MR has shown to be most effective in reducing the risk of secondary cerebral infarction. Lowering total cholesterol further from this level is unlikely to impact on stroke risk to the same extent as combined antiplatelet therapy, although it will impact on future risk of events connected with ischaemic heart disease.

94. A 53-year-old hospital porter is referred to you with aching and fatigue in his legs. His symptoms started 2 months ago with difficulty walking up stairs. On direct questioning, he says it takes him increased effort to get dressed. He also complains of a dry mouth, but there are no speech or swallowing difficulties. On systematic questioning, he has a long history of mild breathlessness on exertion and a chronic cough that he attributes to his heavy smoking. However, over the last 2 weeks he has noticed blood streaks in his sputum. On examination he has a predominantly proximal weakness, more so on the left. Deep tendon reflexes are depressed but improve with repeated muscle contraction. There are no sensory changes or extraocular muscle abnormalities. What is the most likely diagnosis? Paraneoplastic neuropathy Vasculitic neuropathy Metabolic myopathy Tuberculous myeloradiculopathy LambertEaton syndrome Your answer The LambertEaton syndrome is a myasthenic syndrome with a well-recognised association with malignancy (5060% of cases), most commonly small-cell lung carcinoma. It is characterised by proximal muscle weakness, particularly in the legs, which improves for a short while after exercise (postexercise facilitation). Extraocular and bulbar muscles are usually spared. In addition, tendon reflexes are usually depressed or absent and are characteristically enhanced with repeated muscle contraction. Autonomic disturbances (eg dry mouth and impotence, as in the above case) are common. High-frequency repetitive nerve stimulation results in an incremental response of the compound muscle action potential because accumulation of presynaptic calcium facilitates acetylcholine release. The haemoptysis could also have been due to tuberculosis or a systemic

vasculitis, either of which could be associated with neurological features. His signs could be consistent with a patchy mononeuritis multiplex, but Lambert Eaton syndrome is the most likely.

95. A 73-year-old man presents to his GP complaining of a headache and blurring of vision in his right eye lasting for 5 minutes. Two days later he developed sudden-onset, left-sided weakness affecting both upper and lower limbs and lasting for 30 minutes. His past medical history includes mild hypertension. He is a non-smoker. There are no neurological abnormalities. His blood pressure is 160/90 mmHg, heart rate 88 bpm. There are no audible carotid bruits. Which of the following is the most likely diagnosis? Migraine equivalent

Transient ischaemic attack secondary to carotid artery disease Transient ischaemic attack secondary to cardioembolism Transient ischaemic attack secondary to small-vessel disease Transient ischaemic attack secondary to giant-cell arteritis

Your answer

The transient monocular visual disturbance is consistent with the description of amaurosis fugax. Characteristically, patients describe a curtain coming down over their vision, but are just as likely to describe blurring or greying. Any suggestion that this disturbance is altitudinal (ie has a horizontal cut-off) makes the possibility that this is a vascular disturbance of the retinal vessels much greater. The subsequent left-sided weakness is suggestive of a right cerebral hemisphere transient ischaemic attack. The combination of these two strongly points towards a right internal carotid artery stenosis as the source of platelet emboli. Other sources of emboli should be considered. However, cardioembolism is unlikely in the absence of either atrial fibrillation or previous cardiac symptoms (of either cardiac ischaemia or cardiac failure). Transient occlusion of a small penetrating vessel could cause the hemiparesis, but not the amaurosis fugax. Giant-cell arteritis should always be considered in patients over 60 years

of age, but there are no specific features to support the diagnosis, although it is always worthwhile checking the ESR. Lastly, migraine equivalents (aura-like symptoms without headache) can provide diagnostic difficulties, but there is no suggestion of the characteristic slow spread of symptoms in this patient.

96. A 58-year-old farmer from Northern Ireland presents with a progressive neurological illness over 3 weeks. He complains of double vision and has some slurring of speech and unsteadiness on his feet, as well as malaise and tiredness. He also admits to having lost 6.25 kg (1 stone) in weight over a 6-week period. On examination he has a bilateral VIth nerve palsy, facial asymmetry and marked dysarthria. The deep tendon reflexes are sluggish throughout and the plantar responses equivocal. He has lymphadenopathy of the axilla and groins, and a temperature of 37.6 C. Chest X-ray is normal. MRI of the brain shows diffuse meningeal enhancement. Lumbar puncture shows an elevated protein of 1.2 g/l, an elevated white cell count of 87/mm3 (mostly lymphocytes) and a low CSF/blood glucose ratio (CSF glucose 0.2 mmol/l, blood glucose 6.7 mmol/l). Which cause for his symptoms should be considered most likely pending further investigations? Brucellosis Sarcoidosis Malignant meningitis Tuberculous (TB) meningitis Your answer Lyme disease

Meningeal infection with Mycobacterium tuberculosis is notoriously difficult to diagnose and treat. The clues in this case are a typical clinical presentation and

CSF picture, with a very low CSF glucose to blood glucose ratio. Repeated CSF samples (often in largish volumes) are usually required for prolonged culture. It is not uncommon to need to treat a patient for TB meningitis based on reasonable clinical suspicion, without laboratory confirmation. However, such a decision is clearly a difficult one given the length of the treatment required and the potential toxicity of the drugs involved. Brucellosis is a reasonable consideration given his occupation but the CSF glucose would be expected to be normal or only very slightly reduced, as it would in sarcoid and Lyme disease. Malignant meningitis would also be unlikely to produce a glucose level as low as this; in any case treatment for TB would be given until definite evidence of an alternative diagnosis was produced. 97. A 75-year-old man is noted by his family to be increasingly confused. His mental state fluctuates considerably and he appears to be hallucinating at times. His daughter commented that his gait has become abnormal over the preceding months, and that his GP had tried a tablet for the nocturnal confusion that apparently made him much worse. On examination he has a Mini-Mental score of 20/30, has generally increased tone and difficulty in performing rapidly alternating movements. His tendon reflexes are slightly brisk but the plantar responses are normal and no primitive reflexes are detected. Which of the following is the most likely explanation of this clinical scenario? Vascular dementia Senile dementia of Alzheimers type CreutzfeldtJakob disease Temporal variant of frontotemporal dementia Dementia with Lewy bodies

Your answer

The associated extrapyramidal features and variable psychiatric features with complex hallucinations are typical of dementia with Lewy bodies (DLB). This condition is now recognised to be the second most common cause of neurodegenerative disease in older people (after senile dementia of Alzheimers type, SDAT). Typically, standard antipsychotic drugs such as haloperidol worsen DLB, and indeed potentially fatal neuroleptic sensitivity reactions can occur. It is one of a group of conditions caused by abnormal aggregation of the synaptic protein a-synuclein. The cholinesterase inhibitor drugs, initially developed for SDAT, look promising for the treatment of DLB.

98. A 45-year old man complains of numbness of his left thigh. He feels his balance may be poorer than usual but is not sure of this. He has put on 6.25 kg (1 stone) in weight over the past 6 months. He has mild, chronic, low back pain. There is a family history of a neurological condition, in that his fathers brother is said to have multiple sclerosis. On examination he is overweight. He has some reduction of pinprick sensation over the anterior part of his thigh on the left,

with no motor signs. Tendon reflexes are normal. There are no cerebellar signs. What is the most likely diagnosis? Sporadic multiple sclerosis (MS) Familial multiple sclerosis Lumbar canal stenosis with nerve root entrapment Meralgia paraesthetica Your answer Inflammatory disease of the lumbosacral plexus

Meralgia paraesthetica is a symptom complex that includes numbness, paraesthesias and pain in the anterolateral thigh, which may result from either an entrapment neuropathy or a neuroma of the lateral femoral cutaneous nerve (LFCN). Obesity or weight gain is a risk factor for this as it predisposes to nerve compression; individual variation in anatomy around the region of the anterior superior iliac spine may also contribute. The diagnosis is mainly clinical (including the absence of other signs) and electrophysiological studies can help to exclude other more generalised neuroradiculopathy. The family history of MS in this case is a red herring; it should be remembered that even if there are no other clinical features to suggest a particular neurological condition, the patient is likely to be anxious about the possibility and clinicians should be aware of this, paying special attention to answering a patients spoken and unspoken concerns.

99. A 76-year-old man presents with a 1-month history of lower back pain. More recently he has developed weakness of and paraesthesias in his legs. On the day of admission, he was unable to stand, and developed difficulty in initiating micturition. He is a long-standing, insulin-dependent diabetic and his diabetic control poor. His most recent Hb A1c is 8.6%. On examination he is mildly febrile. He has increased tone and pyramidal distribution weakness in his lower limbs. Lower limb reflexes are brisk with extensor plantars. Sensory examination is variable. Straight-leg raising produces pain. What is the most likely diagnosis?

Spinal epidural abscess Your answer Anterior spinal artery thrombosis Spinal arteriovenous malformation Tuberculous meningitis Prolapsed L3/4 intervertebral disc This man clearly has an upper motor neurone lesion, most likely in the spinal cord. Vascular events in the cord (thrombosis or rupture of AVM) are usually sudden and catastrophic. A prolapsed disc at L3/4 is possible, but the history of fever in the context of diabetes should always raise the possibility of infection, eg spinal epidural abscess. He will require an MRI of the spine, blood cultures and probably surgical decompression and intravenous antibiotics.

100. A 49-year-old woman was placed on warfarin therapy because of pulmonary embolism. While she has been on therapy, she has developed pain, numbness and paraesthesias from her left anterior thigh extending to her medial leg, plus weakness of knee extension and hip flexion. The left knee jerk is absent. What is the most likely site of the lesion? Left common peroneal nerve Left femoral nerve Left obturator nerve Left sciatic nerve Left superior gluteal nerve

Your answer

All abnormalities are indicative of a left femoral neuropathy. The patient has weakness of knee extension (quadriceps) and hip flexion (iliopsoas). She has decreased sensation in her anterior thigh (anterior femoral cutaneous nerve) and medial distal leg (saphenous nerve). A patient on anticoagulation therapy can suffer a retroperitoneal haemorrhage, which can damage the femoral nerve.